Posterior reversible encephalopathy syndrome with Lilliputian hallucinations secondary to Takayasu's arteritis

Abstract

Posterior Reversible Encephalopathy Syndrome (PRES) is a rare complication of Takayasu's Arteritis. A 54-year-old, right-handed woman presented with Lilliputian visual hallucinations, postprandial abdominal pain, blurred vision and headaches. She then had a tonic-clonic seizure. Neuroimaging revealed characteristic white matter oedema of the occipital lobes, in keeping with PRES. Renal infarcts and abnormalities of the abdominal aorta, subclavian, mesenteric, and internal carotid arteries were demonstrated on further imaging. The combination of hypertension, absent peripheral pulses, postprandial claudication, and imaging abnormalities of the aorta as well as its branches, lead to the diagnosis of PRES secondary to Takayasu's Arteritis. Treatment with oral steroids resulted in complete resolution of the patient's symptoms and abnormalities found on CT and MRI brain imaging. Takayasu's Arteritis is a rare vasculitis, more common in women and PRES is an unusual complication. Symptoms of PRES may include headache, seizures, hallucinations, confusion, and altered consciousness. Risk factors for PRES include; pregnancy, immunosuppression, renal disease, hypertension and rheumatological disorders. Vasogenic oedema in affected lobes, most often occipital, is characteristic of PRES on neuroimaging. Prompt treatment of PRES can avoid catastrophic consequences such as death and can achieve complete resolution of symptoms and imaging abnormalities.

Cerebrovascular Disease in the Setting of Posterior Reversible Encephalopathy Syndrome

“…Takayasu's arteritis (TA) is a rare chronic inflammatory disease that primarily affects the aorta, aortic branches, and the pulmonary artery ( 10 ). PRES secondary to TA has been reported in 13 patients, according to a PubMed search ( 10 , 20 ). In addition to TA secondary to PRES, in isolated cases, PRES has also been reported to occur secondary to rare vasculitis, such as cerebral amyloid angiopathy-related inflammation ( 21 ), and antineutrophil cytoplasmic antibody-associated vasculitis ( 22 ).…”

“…The main pathophysiological change in TA is panarteritis involving all vessel wall layers. In the acute phase, production of inflammatory cytokines and mediators induces continuous endothelial injury, while in the chronic phase, hyperplasia of the vessel wall and fibrosis of the arterial wall cause luminal narrowing and can lead to the development of hypertension ( 20 ). Endothelial injury and hypertension in patients with TA make this disease an ideal environment for PRES development.…”

“…PRES secondary to TA presents as fluid-attenuated inversion recovery hyperintensities on MRI, while there is usually no diffusion restriction on DWI ( 10 , 20 ). The onset could be in the bilateral parieto-occipital lobes, bilateral temporoparietal-occipital lobes, and the cerebellum, as reported for PRES caused by other factors ( 10 , 20 ).…”

“…PRES secondary to TA presents as fluid-attenuated inversion recovery hyperintensities on MRI, while there is usually no diffusion restriction on DWI ( 10 , 20 ). The onset could be in the bilateral parieto-occipital lobes, bilateral temporoparietal-occipital lobes, and the cerebellum, as reported for PRES caused by other factors ( 10 , 20 ). In addition, after CTA or MRA, TA should be considered when combined intracranial artery stenosis, intracranial aneurysm, carotid artery occlusion, and other vascular changes are found.…”